Product Description

ABCBB Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES9453-50, ES9453-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MDR/TAP subfamily. Members of the MDR/TAP subfamily are involved in multidrug resistance. The protein encoded by this gene is the major canalicular bile salt export pump in man. Mutations in this gene cause a form of progressive familial intrahepatic cholestases which are a group of inherited disorders with severe cholestatic liver disease from early infancy. [provided by RefSeq, Jul 2008],

Reactivity: Human, Rat, Mouse,

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from part region of human protein

Isotype: IgG

Target: ABCBB

Specificity: ABCBB Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Expression: Expressed predominantly, if not exclusively in the liver, where it was further localized to the canalicular microvilli and to subcanalicular vesicles of the hepatocytes by in situ,

Observed Band (KD): 145kD

Fields: Endocrine resistance, ABC transporters, Bile secretion, Cholesterol metabolism

Function: Disease: Defects in ABCB11 are the cause of benign recurrent intrahepatic cholestasis type 2 (BRIC2) [MIM: 605479]. BRIC is characterized by intermittent episodes of cholestasis without progression to liver failure. There is initial elevation of serum bile acids, followed by cholestatic jaundice which generally spontaneously resolves after periods of weeks to months. The cholestatic attacks vary in severity and duration and patients are asymptomatic between episodes, both clinically and biochemically
Disease: Defects in ABCB11 are the cause of progressive familial intrahepatic cholestasis type 2 (PFIC2) [MIM: 601847]. PFIC2 is an inherited liver disease of childhood which is characterized by cholestasis and normal serum gamma-glutamyltransferase activity. Defects in ABCB11 are also found in cases of chronic intrahepatic cholestasis without obvious familial history of chronic liver disease

Subcellular Location: Apical cell membrane, Multi-pass membrane protein Recycling endosome membrane, Multi-pass membrane protein Endosome Cell membrane, Multi-pass membrane protein Internalized at the canalicular membrane through interaction with the adapter protein complex 2 (AP-2) (PubMed:22262466) At steady state, localizes in the canalicular membrane but is also present in recycling endosomes ABCB11 constantly and rapidly exchanges between the two sites through tubulo-vesicles carriers that move along microtubules Microtubule-dependent trafficking of ABCB11 is enhanced by taurocholate and cAMP and regulated by STK11 through a PKA-mediated pathway Trafficking of newly synthesized ABCB11 through endosomal compartment to the bile canalicular membrane is accelerated by cAMP but not by taurocholat

Storage and Stability: -20°C/1 year

Gene Name: ABCB11 BSEP

Protein Name: Bile salt export pump (ATP-binding cassette sub-family B member 11)

Mouse SwissProt Number: Q9QY30

Rat SwissProt Number: O70127

Research Use Only